The heartbreaking disease

Kawasaki Disease has been baffling doctors since it was first recorded in Australia in the 1970's. Faye Triantafillopoulos shares her experience as a mother of a child effected by this rare but life threatening condition

Faye Triantafillopoulos will never forget the Easter Sunday two years ago when her daughter Anastasia became ill.

“She had a fever and complained of a burning tummy, as the day progressed it got worse,” says Faye, “I thought it was just a virus, then the following day, Anastasia said her fingertips were hurting. She was putting them in her mouth to relieve them, which I thought was very unusual.”

“As a mother you don’t want to panic,” says Faye, who also saw a rash appear on her daughter’s chest.

Faye decided within hours to take Anastasia to the emergency department of Melbourne’s Royal Children’s Hospital. They suggested waiting it out. It was probably a virus, nothing to worry about, so Faye took her daughter home.

The next day, with Anastasia’s temperature soaring to 40 degrees, they were back at RCH. After keeping the ailing four-year-old in for observation, and completing a rigorous series of tests that ruled out the usual ailments, finally the doctors agreed: the cause of Anastasia’s distress was Kawasaki Disease (KD).

Extremely difficult to diagnose, KD is thankfully rare. The condition that can result in damage to the coronary arteries is most commonly found in children under five years of age, though incidences have been known in older children.

Early diagnosis of the disease, which is likely to occur in around 200 Australian children a year, can mean a full recovery. But if its symptoms are misdiagnosed, the condition can be deadly.

Anastasia was fortunate. Early detection meant that she was able to receive a transfusion of immunoglobulin before the ‘sub-acute’ phase of the disease, the period when the most severe coronary damage is done.

Dr David Burgner is Australia’s leading specialist in the study of Kawasaki Disease. Working out of the Murdoch Children’s Research Institution in Melbourne, Dr Burgner told Neos Kosmos that the condition, first documented in the late 1960s in Japan by Dr Tomisaku Kawasaki, continues to baffle the medical profession worldwide.

“It wasn’t recognised here until the late 1970s and even now it’s likely that many cases are missed,” says Dr Burgner, who has been studying the disease for 13 years and is involved with researchers in 16 countries in a global effort to understand the condition better.

Though paediatricians are now switched onto KD as a possible diagnosis, Dr Burgner points out that for a GP, who may have never come across the condition previously, it’s a different matter.

“It’s the great pretender, in that a child can have one or two of the symptoms but still develop the most serious complication – damage to the coronary arteries.”

Without treatment the patient will be acutely unwell for two or three weeks, then have, a ‘sub-acute’ phase. “That’s when we think the damage occurs,” says Dr Burgner.

“KD causes inflammation of the blood vessels throughout the body, but has a particular tendency to pick off the coronary arteries that supply blood to the heart. ”

What makes the currently prescribed treatment effective, is as mysterious as trying to diagnose the disease, says Dr Burgner.

“The treatment is intravenous immunoglobulin transfusion, which is antibodies from a blood donor. Untreated, about a quarter of children who contract the disease will have damage to their coronary arteries. With treatment, this is reduced to about five per cent of children. But we don’t understand exactly know how the immunoglobulin is working.

“We try to treat within the first ten days. Children who have been severely affected will often need to be on lifelong medical therapy, and sometimes they need to have a coronary artery bypass.”

Dr Burgner’s says that knowing what causes KD, continues to elude the medical community.

“Some think it’s a virus, others that it’s a bacteria. There are a hundred different theories out there. “We do know that the disease is an abnormal immune response that some children have to infection. All children are exposed to this trigger, but only some will over react.”

With the disease 20 times more common in Japanese children compared to white Caucasian society, it’s clear that KD has a distinct genetic dimension says Dr Burgner.

“It’s something about their genetic make-up that makes them more prone to develop it. The genetics are our best hope in trying to understand this complicated disease.”

Two years on Anastasia Triantafillopoulos is ok. “The tests she’s been having since, show there hasn’t been any damage to her heart,” says her mum. “She’s fine because we got it so early. I was very lucky.”

For further information on Kawasaki Disease go to www.kdfoundation.org.au